Répéter s'il vous plait: Working memory intensive sentence repetition deficits as a sensitive neuropsychological marker of primary progressive aphasia

ABSTRACT. The differential diagnosis of primary progressive aphasia (PPA) is challenging due to overlapping clinical manifestations of the different variants of the disease. This is particularly true for the logopenic variant of PPA (lvPPA), in which such overlap was reported with regard to impairments in repetition abilities. In this study, four individuals with lvPPA underwent standard neuropsychological and language assessments. The influence of psycholinguistic variables on their performance of in word, nonword and sentence repetition tasks was also specifically explored. Some level of heterogeneity was found in cognitive functions and in language. The four participants showed impairment in sentence repetition in which their performance was negatively affected by semantic reversibility and syntactic complexity. This study supports the heterogeneity of lvPPA with respect to the cognitive and linguistic status of participants. It also shows that sentence repetition is influenced not only by length, but also by semantic reversibility and syntactic complexity, two psycholinguistic variables known to place additional demands on phonological working memory.

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A Case of a Monolingual Spanish-Speaking Latina with Semantic Variant Primary Progressive Aphasia (svPPA)

Archives of Clinical Neuropsychology ◽

10.1093/arclin/acz029.57 ◽

2019 ◽

Vol 34 (7) ◽

pp. 1290-1290

Author(s):

L Perez

Keyword(s):

Working Memory ◽

Language Production ◽

Primary Progressive Aphasia ◽

Motor Speech ◽

Problem Solving Skills ◽

Progressive Aphasia ◽

Primary Progressive ◽

Spanish Speaking ◽

Word Finding ◽

Semantic Variant

Abstract Objective Often, individuals with lower educational attainment and limited proficiency in the English language get misdiagnosed and/or undertreated, which can impact their quality of life and other outcomes. The present case study intends to review and discuss the presentation of a monolingual, Spanish-speaking woman with Semantic Variant Primary Progressive Aphasia (svPPA), who was originally referred for a neuropsychological evaluation to determine the severity of her existing Alzheimer’s Disease (AD) diagnosis. Case Description Ms. X, is a 64-year-old, right-handed Hispanic woman with 6 years of education. Symptoms included forgetfulness, restlessness, and insomnia. Her family reported that she was repeating her ideas frequently during conversations and failing to recognize previously acquainted people, including her own relatives. A recent MRI of the brain showed anterior temporal lobe atrophy. Diagnostic Impressions and Outcomes Overall, she showed naming deficits (anomia), impaired verbal fluency, surface dyslexia, and significant problems with comprehension. Executive functioning, sentence repetition, working memory, and attention were generally intact. Qualitatively, her speech was apparently fluent and automatic, yet clearly empty in meaning. In Ms. X’s case, collateral reports of word-finding difficulties, tendency to repeat her thoughts incessantly, associative agnosia and prosopagnosia, and spared repetition and motor speech are strongly indicative of svPPA. Discussion svPPA primarily impacts language production and comprehension, and is characterized by severe anomia, word-finding difficulties, impaired single word comprehension, and in some cases, defective recognition of familiar faces. On testing, impairments can be observed in confrontation naming, with motor speech and repetition, working memory, episodic memory, visuospatial skills, and problem-solving skills relatively intact. Language symptoms are thought to stem from deficits of the semantic system.

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Neural substrates of verbal repetition deficits in primary progressive aphasia

Brain Communications ◽

10.1093/braincomms/fcab015 ◽

2021 ◽

Author(s):

Hilary E Miller ◽

Claire Cordella ◽

Jessica A Collins ◽

Rania Ezzo ◽

Megan Quimby ◽

...

Keyword(s):

Working Memory ◽

Cortical Thickness ◽

Digit Span ◽

Primary Progressive Aphasia ◽

Supramarginal Gyrus ◽

Phonological Working Memory ◽

Progressive Aphasia ◽

Primary Progressive ◽

Left Posterior ◽

Verbal Repetition

Abstract In this cross-sectional study, we examined the relationship between cortical thickness and performance on several verbal repetition tasks in a cohort of patients with primary progressive aphasia in order to test predictions generated by theoretical accounts of phonological working memory that predict phonological content buffers in left posterior inferior frontal sulcus and supramarginal gyrus. Cortical surfaces were reconstructed from magnetic resonance imaging scans from 42 participants diagnosed with primary progressive aphasia. Cortical thickness was measured in a set of anatomical regions spanning the entire cerebral cortex. Correlation analyses were performed between cortical thickness and average score across three phonological working memory related tasks: the Repetition subtest from the Western Aphasia Battery, a forward digit span task, and a backward digit span task. Significant correlations were found between average working memory score across tasks and cortical thickness in left supramarginal gyrus and left posterior inferior frontal sulcus, in support of prior theoretical accounts of phonological working memory. Exploratory whole-brain correlation analyses performed for each of the three behavioral tasks individually revealed a distinct set of positively-correlated regions for each task. Comparison of cortical thickness measures from different primary progressive aphasia subtypes to cortical thickness in age-matched controls further revealed unique patterns of atrophy in the different subtypes.

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Episodic and working memory function in Primary Progressive Aphasia: A meta-analysis

Neuroscience & Biobehavioral Reviews ◽

10.1016/j.neubiorev.2018.06.015 ◽

2018 ◽

Vol 92 ◽

pp. 243-254 ◽

Cited By ~ 13

Author(s):

Willem S. Eikelboom ◽

Nikki Janssen ◽

Lize C. Jiskoot ◽

Esther van den Berg ◽

Ardi Roelofs ◽

...

Keyword(s):

Working Memory ◽

Meta Analysis ◽

Memory Function ◽

Primary Progressive Aphasia ◽

Progressive Aphasia ◽

Primary Progressive

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Sentence repetition impairment in all variants of primary progressive aphasia

Frontiers in Human Neuroscience ◽

10.3389/conf.fnhum.2017.223.00019 ◽

2017 ◽

Vol 11 ◽

Author(s):

Naida Graham ◽

David Tang-Wai ◽

Sandra Black ◽

Carol Leonard ◽

Mario Masellis ◽

...

Keyword(s):

Primary Progressive Aphasia ◽

Progressive Aphasia ◽

Primary Progressive ◽

Sentence Repetition

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Profiling sentence repetition deficits in primary progressive aphasia and Alzheimer’s disease: Error patterns and association with digit span

Brain and Language ◽

10.1016/j.bandl.2019.03.001 ◽

2019 ◽

Vol 194 ◽

pp. 1-11 ◽

Cited By ~ 2

Author(s):

Ashleigh Beales ◽

Anne Whitworth ◽

Jade Cartwright ◽

Peter K. Panegyres ◽

Robert T. Kane

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Digit Span ◽

Primary Progressive Aphasia ◽

Progressive Aphasia ◽

Error Patterns ◽

Primary Progressive ◽

Sentence Repetition

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Clinical marker for Alzheimer disease pathology in logopenic primary progressive aphasia

Neurology ◽

10.1212/wnl.0000000000004034 ◽

2017 ◽

Vol 88 (24) ◽

pp. 2276-2284 ◽

Cited By ~ 45

Author(s):

Lucia A.A. Giannini ◽

David J. Irwin ◽

Corey T. McMillan ◽

Sharon Ash ◽

Katya Rascovsky ◽

...

Keyword(s):

Alzheimer Disease ◽

Digit Span ◽

Primary Progressive Aphasia ◽

Angular Gyrus ◽

Lexical Retrieval ◽

Central Feature ◽

Retrospective Case ◽

Progressive Aphasia ◽

Primary Progressive ◽

Sentence Repetition

Objective:To determine whether logopenic features of phonologic loop dysfunction reflect Alzheimer disease (AD) neuropathology in primary progressive aphasia (PPA).Methods:We performed a retrospective case-control study of 34 patients with PPA with available autopsy tissue. We compared baseline and longitudinal clinical features in patients with primary AD neuropathology to those with primary non-AD pathologies. We analyzed regional neuroanatomic disease burden in pathology-defined groups using postmortem neuropathologic data.Results:A total of 19/34 patients had primary AD pathology and 15/34 had non-AD pathology (13 frontotemporal lobar degeneration, 2 Lewy body disease). A total of 16/19 (84%) patients with AD had a logopenic spectrum phenotype; 5 met published criteria for the logopenic variant (lvPPA), 8 had additional grammatical or semantic deficits (lvPPA+), and 3 had relatively preserved sentence repetition (lvPPA−). Sentence repetition was impaired in 68% of patients with PPA with AD pathology; forward digit span (DF) was impaired in 90%, substantially higher than in non-AD PPA (33%, p < 0.01). Lexical retrieval difficulty was common in all patients with PPA and did not discriminate between groups. Compared to non-AD, PPA with AD pathology had elevated microscopic neurodegenerative pathology in the superior/midtemporal gyrus, angular gyrus, and midfrontal cortex (p < 0.01). Low DF scores correlated with high microscopic pathologic burden in superior/midtemporal and angular gyri (p ≤ 0.03).Conclusions:Phonologic loop dysfunction is a central feature of AD-associated PPA and specifically correlates with temporoparietal neurodegeneration. Quantitative measures of phonologic loop function, combined with modified clinical lvPPA criteria, may help discriminate AD-associated PPA.

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